Richards James, Wei Rui, Anjum Fatima
Musgrove Park Hospital
Temple University, Lewis Katz School of Medicine
Acute esophageal necrosis (AEN), also referred to as black esophagus, Gurvits syndrome, or acute necrotizing esophagitis, is a rare and life-threatening condition affecting the esophagus. This condition is characterized by either partial or total circumferential blackening of the esophagus that stops abruptly at the gastroesophageal junction, as observed on an upper gastrointestinal endoscopy. AEN was first documented in 1990 by Goldberg et al, and the exact cause remains unclear, but AEN tends to follow a pattern of acute ischemia associated with a topical insult to the esophagus. AEN has a prevalence of up to 0.2% in the autopsy series and ranges from 0.01% to 0.28% in the endoscopy series. This condition occurs more frequently in males, with an incidence up to 4 times higher compared to that in females, and typically affects middle-aged individuals. AEN predominantly impacts the distal esophagus, affecting 97% of cases. AEN typically spares the proximal two-thirds of the esophagus and almost always stops at the gastroesophageal junction. Management of AEN is primarily medical but can include surgical intervention in the event of complications such as perforation and mediastinitis. Overall mortality associated with AEN is approximately 30%; however, mortality specific to AEN is closer to 5%.
急性食管坏死（AEN），也被称为黑色食管、古维茨综合征或急性坏死性食管炎，是一种影响食管的罕见且危及生命的疾病。这种疾病的特征是在食管上消化道内镜检查中观察到食管出现部分或全周性变黑，且在胃食管交界处突然停止。AEN于1990年首次由戈德堡等人记录，确切病因尚不清楚，但AEN往往遵循与食管局部损伤相关的急性缺血模式。在尸检系列中，AEN的患病率高达0.2%，在内镜检查系列中为0.01%至0.28%。这种疾病在男性中更常见，发病率比女性高4倍，通常影响中年个体。AEN主要影响食管远端，97%的病例受此影响。AEN通常不累及食管近端的三分之二，几乎总是在胃食管交界处停止。AEN的治疗主要是药物治疗，但如果出现穿孔和纵隔炎等并发症，可能包括手术干预。与AEN相关的总体死亡率约为30%；然而，AEN特有的死亡率接近5%。
